Ectopic adrenocortical adenoma characterized by hypogonadism: a case report and review of the literature.

BACKGROUND: Currently, there is a scarcity of cases and diagnostic data regarding ectopic adrenocortical adenomas, particularly in relation to their impact on gonadal function and localization diagnostic techniques. We report a typical case of ectopic adrenocortical adenomas and the data of treatment follow-up, and review the literature of 31 available cases of ectopic adrenocortical adenomas. CASE PRESENTATION: A 27-year-old Chinese female patient was admitted to our hospital for hypertension, hyperglycaemia and primary amenorrhea. The patient was functionally diagnosed with ACTH-independent CS and hypogonadotropic hypogonadism. Radiological evaluations, including Computed Tomography (CT) and functional imaging, identified a mass at the left renal hilum. Histological assessments post-surgical excision confirmed the mass to be an ectopic adrenocortical adenoma. A subsequent 3-month follow-up showed no signs of disease recurrence, a swift recovery of the cortisol axis was observed, with a partial recuperation of the gonadal axis. REVIEW: Our literature review shows that the most common ectopic areas of cortisol adenomas are renal hilum and hepatic region. The most positive biomarker is Melan A, and only a few cases have been diagnosed with functional localization. CONCLUSION: Ectopic adrenocortical adenomas may be asymptomatic in the early stage and can impact gonadal function. Physicians who treat hypogonadism must be aware of the need to test cortisol levels and perform functional localization in patients with lumps present.

Oncocytic adrenal cortical adenoma: a benign lesion mimicking malignancy.

Adrenocortical tumours are rare in children and account for only 0.3%-0.4% of all neoplasms in childhood. They present with variable signs and symptoms, depending on the type of hormonal hypersecretion. The majority of the adrenocortical tumours in children are functional (90%) and malignant (88%). Here, we describe a functional plurihormonal oncocytic adrenal cortical adenoma in a young girl, that mimicked a malignant adrenal lesion, clinically as well as on imaging and biochemical features. This report bears the objective of being aware of the atypical biochemical as well as imaging characteristics of oncocytic adrenal tumours.

Fully-automated CT derived body composition analysis reveals sarcopenia in functioning adrenocortical carcinomas.

Determination of body composition (the relative distribution of fat, muscle, and bone) has been used effectively to assess the risk of progression and overall clinical outcomes in different malignancies. Sarcopenia (loss of muscle mass) is especially associated with poor clinical outcomes in cancer. However, estimation of muscle mass through CT scan has been a cumbersome, manually intensive process requiring accurate contouring through dedicated personnel hours. Recently, fully automated technologies that can determine body composition in minutes have been developed and shown to be highly accurate in determining muscle, bone, and fat mass. We employed a fully automated technology, and analyzed images from a publicly available cancer imaging archive dataset (TCIA) and a tertiary academic center. The results show that adrenocortical carcinomas (ACC) have relatively sarcopenia compared to benign adrenal lesions. In addition, functional ACCs have accelerated sarcopenia compared to non-functional ACCs. Further longitudinal research might shed further light on the relationship between body component distribution and ACC prognosis, which will help us incorporate more nutritional strategies in cancer therapy.

Adrenocortical Adenoma Arising from Adrenohepatic Fusion: A Mimic of Hepatocellular Carcinoma-Case Report.

AIM: We present a case of adrenocortical adenoma originating from the adrenohepatic fusion (AHF) region, accompanied by advanced hepatosteatosis in the liver tissue, and discuss its distinction from hepatocellular carcinoma.  Case Experience: A 68-year-old male patient was admitted to the hospital following a fall from a height. He was referred to our hospital after an incidental discovery of a liver mass during an abdominal ultrasound examination. Subsequently, magnetic resonance imaging (MRI) imaging was conducted, followed by segmental liver resection with right adrenalectomy, and histological analysis of a biopsy from the lesion.  Results: Upon histologic examination, the case was determined to be an adrenocortical adenoma originating from the AHF.  Discussion: Adrenohepatic fusion (AHF) denotes the histological amalgamation of cells from the right adrenal cortex and right hepatic parenchyma. Only a limited number of cases of neoplasia originating from this region have been documented. These rare instances often present a diagnostic challenge, with preoperative imaging frequently misidentifying them as primary malignancies of either hepatic or adrenal origin, potentially leading to unnecessary extensive resections. The integration of immunohistochemical staining alongside clinical and radiological data proves helpful for accurately diagnosing this condition.  Conclusion: Awareness among clinicians, radiologists, and pathologists regarding the tumors that may arise from this region can mitigate the risk of performing extensive resections unnecessarily.

Contrast CT radiomic features add value to prediction of prognosis in adrenal cortical carcinoma.

OBJECTIVE: Adrenocortical carcinoma (ACC) is a rare and aggressive malignancy with poor prognosis due to high postoperative recurrence rates. The aim of this study is to develop a contrast CT radiomic feature-based prognosis prediction model for ACC and evaluate its performance by comparison with ENSAT staging system and S-GRAS score. METHODS: Included in this study were 39 ACC patients, from which we extracted 1411 radiomic features. Using cross-validated least absolute shrinkage and selection operator regression (cv-LASSO regression), we generated a radiomic index. Additionally, we further validated the radiomic index using both univariate and multivariate Cox regression analyses. We constructed a radiomic nomogram that incorporated the radiomic signature and compared it with ENSAT stage and S-GRAS score in terms of calibration, discrimination and clinical usefulnes. RESULTS: In this study, the average progression free survival (PFS) of 39 patients was 20.4 (IQR 9.1-60.1) months and the average overall survival (OS) was 57.8 (IQR 32.4-NA). The generated radiomic features were significantly associated with PFS, OS, independent of clinical-pathologic risk factors (HR 0.16, 95%CI 0.02-0.99, p = 0.05; HR 0.20, 95%CI 0.04-1.07, p = 0.06, respectively). The radiomic index, ENSAT stage, resection status, and Ki67% index incorporated nomogram exhibited better performance for both PFS and OS prediction as compared with the S-GRAS and ENSAT nomogram (C-index: 0.75 vs. C-index: 0.68, p = 0.030 and 0.67, p = 0.025; C-index: 0.78 vs. C-index: 0.72, p = 0.003 and 0.73, p = 0.006). Calibration curve analysis showed that the radiomics-based model performs best in predicting the two-year PFS and the three-year OS. Decision curve analysis demonstrated that the radiomic index nomogram outperformed the S-GRAS and ENSAT nomogram in predicting the two-year PFS and the three-year OS. CONCLUSION: The contrast CT radiomic-based nomogram performed better than S-GRAS or ENSAT in predicting PFS and OS in ACC patients.

Right Adrenocortical Carcinoma Coexisting With Left Adrenal Sarcomatoid Carcinoma on FDG PET/CT.

ABSTRACT: Bilateral adrenal glands synchronously involved by different types of pathologies are uncommon. An 80-year-old man underwent FDG PET/CT to evaluate bilateral adrenal masses, which were initially discovered by ultrasonography and confirmed by MRI. The images demonstrated elevated FDG activity in both lesions, which were subsequently diagnosed as concurrent right adrenocortical carcinoma and left adrenal sarcomatoid carcinoma respectively by histopathological examination.

[A Case of Aldosterone-Producing Adrenocortical Carcinoma].

A 59-year-old man presented with high blood pressure, hypokalemia and muscle weakness. His aldosterone/renin ratio was high and plasma renin activity was low. Computed tomography (CT) showed a heterogeneous left adrenal mass. Primary aldosteronism was diagnosed and laparoscopic left adrenalectomy was performed. The pathological diagnosis was adrenocortical carcinoma with positive surgical margins. He underwent radiotherapy and mitotane as adjuvant therapies. Subsequently, CT revealed multiple metastases, in the liver and retroperitoneum. After six courses of EDP (a combination of etoposide, doxorubicin and cisplatin), CT showed widespread metastases in the retroperitoneum and he chose to receive the best supportive care. Aldosterone-producing adrenocortical carcinoma is exceedingly rare. To the best of our knowledge, only67 cases have been reported. Complete resection is needed to improve prognosis and this was not achieved in our case. We therefore recommend careful selection of the operative procedure.

Positive Correlation Between 18 F-FDG Uptake and Tumor-Proliferating Antigen Ki-67 Expression in Adrenocortical Carcinomas.

PURPOSE OF THE REPORT: Adrenocortical carcinoma (ACC) is an extremely rare endocrine malignancy, which cannot always be diagnosed during conventional radiology and hormonal investigations. 18 F-FDG PET could help predict malignancy, but more data are necessary to support future guidelines. METHODS: A cohort of 63 patients with histologically proven ACC (n = 55) or metastatic ACC with steroid oversecretion (n = 8) was assembled. All patients underwent an 18 F-FDG PET, and the SUV max and the adrenal-to-liver SUV max ratio were calculated. The 18 F-FDG PET parameters were compared with clinical, pathological, and outcome data. RESULTS: Fifty-six of 63 patients (89%) had an ACC with an adrenal-to-liver SUV max ratio >1.45, which was a previously defined cutoff value to predict malignancy with 100% sensitivity. Seven ACCs (11%) had a lower uptake (adrenal-to-liver SUV max <1.45), most of them with a proliferation marker Ki-67 expression level <10%. A positive correlation between 18 F-FDG PET parameters (SUV max and adrenal-to-liver SUV max ratio) and tumor size, ENSAT (European Network for the Study of Adrenal Tumors) staging, total Weiss score, and the Ki-67 was found. The strong correlation between SUV max and Ki-67 ( r = 0.47, P = 0.0009) suggests a relationship between 18 F-FDG uptake levels and tumor proliferation. No statistically significant associations between outcome parameters (progression-free or overall survival) and 18 F-FDG PET parameters were found. CONCLUSIONS: This large cohort study shows that most cases of ACC demonstrate high 18 F-FDG uptake. However, the positive correlation observed between SUV max and Ki-67 expression levels seems to explain the possibility of identifying some ACC with a low or inexistent 18 F-FDG uptake. These findings have practical implications for the management of patients with an adrenal mass.

Superiority of 18 F-FDG PET/CT to 68 GA-FAPI PET/CT in Adrenocortical Tumor Imaging.

ABSTRACT: 18 F-FDG PET/CT was performed to evaluate the response to treatment in a 62-year-old female patient who was operated for adrenocortical carcinoma. High FDG uptake was observed in recurrent lesion in the left adrenal gland site and metastatic lesions. In the 68 Ga-FAPI-04 PET/CT study, low FAPI uptake was observed in both recurrences and metastases. In this case, we demonstrated the superiority of 18 F-FDG PET/CT over 68 Ga-FAPI-04 PET/CT in the evaluation of adrenocortical carcinoma.

Adrenocortical carcinoma with venous tumor invasion: is there a role for mini-invasive surgery?

OBJECTIVE: This study aims to investigate early oncologic outcomes in patients with adrenocortical carcinoma (ACC) with venous invasion (VI) treated using both open and mini-invasive approaches. PATIENTS AND MATERIALS: We conducted a retrospective analysis of 4 international referral center databases, including all the patients undergoing adrenalectomy for ACC with VI from January 2007 to March 2020. According to CT scan or MRI, the tumor thrombus was classified into four levels: (1) adrenal vein invasion; (2) renal vein invasion; (3) infra-hepatic Inferior vena cava (IVC); and (4) retro-hepatic IVC. In addition, we divided our patients into patients who had undergone open surgery and mini-invasive surgery. RESULTS: We identified 20 patients with a median follow-up of 12 months. The median tumor size was 110mm. ENSAT stage was II in 4 patients, III in 13 patients, and IV in 3 patients. Tumor thrombus extended in the adrenal vein (n=5), renal vein (n=1), infra-hepatic IVC (n=9), or into the retro-hepatic IVC (n=5). Ten patients were treated with a mini-invasive approach. The patient treated with an open approach reported a more aggressive disease. The two groups did not differ in surgical margins, surgical time, blood losses, complications, and length of stay. The prognosis resulted worse in the patient undergoing open. Kaplan-Meier analysis indicated a difference in OS for the patients stratified by ENSAT stage (Log-rank p=0.011); we also reported a difference in DFS for patients stratified for thrombus extension (p=0.004) and ENSAT stage (p<0.001). CONCLUSION: The DFS of patients with VI from ACC is influenced by the staging and the extension of the venous invasion; the staging influences the OS. The mini-invasive approach seems feasible in selected patients; however, further studies investigating the oncological outcomes are needed. A mini-invasive approach for adrenal tumors with venous invasion is an explorable option in very selected patients.

68Ga-DOTAGA-IAC PET/CT for Imaging Metastatic and Recurrent Adrenocortical Carcinoma: A Case Series.

ABSTRACT: Adrenocortical carcinoma (ACC) is a rare malignancy with a prevalence of 1 to 2 cases/million/year. The diagnosis depends upon endocrine workup followed by imaging with CT, MRI, and 18F-FDG PET/CT. The treatment includes surgical resection, debulking surgery, chemotherapy, and radiotherapy. However, patients do not respond well to any of the available therapies. We present noninvasive imaging of histopathology-proven ACC patients using 68Ga-DOTAGA-IAC PET/CT, specific for integrin αvß3. 68Ga-DOTAGA-IAC PET/CT 45 minutes after IV injection showed a decent tumor-to-background ratio and could be used as a promising radiotracer for metastatic and recurrent ACC.

Differentiating between adrenocortical carcinoma and lipid-poor cortical adenoma: A novel cross-sectional imaging-based score.

BACKGROUND: Discrimination between adrenocortical carcinoma and lipid-poor cortical adenoma preoperatively is frequently difficult as these two entities have overlapping imaging characteristics. Differentiation will allow for the selection of the most appropriate operative approach and may help prevent over-treatment. We aimed to identify imaging features that could preoperatively differentiate adrenocortical carcinoma from lipid-poor cortical adenoma and use them in a novel imaging-based score. METHODS: We conducted a retrospective analysis of patients with pathologically proven adrenocortical carcinoma and lipid-poor cortical adenoma who underwent resection in a single tertiary referral center between March 1998 and August 2020. The inclusion criteria were diameter >1 cm, attenuation >10 Hounsfield units on nonenhanced computed tomography, and histopathologic diagnosis. Patients with metastatic or locally advanced adrenocortical carcinoma adenoma (stages 3-4) were excluded. We developed a score using binary logistic multivariate regression model in 5-fold derivation (∼70%) cohorts with stepwise backward conditional regression as feature selection. Standardized mean regression weight was used as variable score points. RESULTS: We identified 232 adrenals resected across 211 patients. By comparing the imaging characteristics of adrenocortical carcinoma (n = 56) and lipid-poor cortical adenoma (n = 156), we revealed statistically significant differences between the groups in 9 parameters: size, attenuation, thin and thick rim enhancement patterns, heterogeneity, calcification, necrosis, fat infiltration, and lymph node prominence. The score mean performance was 100% sensitivity for the exclusion of adrenocortical carcinoma, 80% specificity (95% confidence interval, 68.3-91.5), 66% positive predictive value (95% confidence interval, 52.3-78.7), and 100% negative predictive value with area under the curve of 0.974. CONCLUSION: We defined and evaluated a novel 9-variable, imaging-based score. This score outperformed any single variable and could facilitate safe preoperative discrimination of adrenocortical carcinoma and lipid-poor cortical adenoma.